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Severity of cutaneous findings predict the presence of systemic symptoms in pediatric maculopapular cutaneous mastocytosis.
[cutaneous mastocytosis]
Although
the
prognosis
of
maculopapular
cutaneous
mastocytosis
(
MPCM
)
,
also
referred
to
as
urticaria
pigmentosa
,
is
often
benign
,
clinicians
lack
evidence
to
reliably
predict
those
at
risk
of
associated
systemic
manifestations
.
We
sought
to
elucidate
clinical
markers
of
disease
severity
to
provide
better
treatment
and
prognostic
information
for
individuals
with
MPCM
.
A
retrospective
chart
review
querying
characteristics
of
children
diagnosed
with
MPCM
in
the
Emory
Dermatology
Clinic
was
performed
.
Follow-up
was
obtained
through
a
clinical
encounter
or
telephone
interview
.
Linear
regression
was
used
to
determine
predictors
of
the
number
of
MPCM-related
systemic
symptoms
.
Of
67
subjects
,
57
%
were
male
,
and
the
mean
age
of
onset
was
4
.
5
months
.
The
maximum
number
of
MPCM
lesions
was
1
to
10
in
16
%
,
11
to
30
in
33
%
,
31
to
50
in
25
%
,
51
to
100
in
6
%
,
and
more
than
100
in
20
%
of
subjects
.
For
their
MPCM
lesions
,
46
%
of
subjects
reported
itching
,
34
%
flushing
,
and
25
%
blistering
.
Reported
systemic
symptoms
included
diarrhea
(
22
%
)
,
abdominal
pain
(
15
%
)
,
wheezing
or
dyspnea
(
13
%
)
,
vomiting
(
10
%
)
,
bone
pain
(
10
%
)
,
headaches
(
8
%
)
,
cough
(
10
%
)
,
rhinorrhea
(
8
%
)
,
irritability
(
6
%
)
,
and
anaphylaxis
(
1
.
5
%
)
.
In
a
multivariate
linear
regression
analysis
,
the
maximum
number
of
MPCM
lesions
(
p
=
0
.
02
)
and
the
number
of
skin
symptoms
(
p
<
0
.
01
)
were
statistically
significant
predictors
of
the
number
of
systemic
symptoms
,
controlling
for
age
of
onset
,
body
sites
involved
,
and
sex
.
The
correlation
between
cutaneous
findings
and
symptomatology
could
aid
clinicians
in
identifying
individuals
with
MPCM
who
might
warrant
systemic
evaluation
and
therapy
.
Diseases
Validation
Diseases presenting
"abdominal pain"
symptom
22q11.2 deletion syndrome
adrenal incidentaloma
alpha-thalassemia
benign recurrent intrahepatic cholestasis
carcinoma of the gallbladder
child syndrome
cholangiocarcinoma
congenital diaphragmatic hernia
cushing syndrome
cutaneous mastocytosis
cystinuria
dedifferentiated liposarcoma
erdheim-chester disease
erythropoietic protoporphyria
fabry disease
familial hypocalciuric hypercalcemia
familial mediterranean fever
focal myositis
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
pleomorphic liposarcoma
primary effusion lymphoma
primary hyperoxaluria type 1
proteus syndrome
scrub typhus
sneddon syndrome
systemic capillary leak syndrome
typhoid
von hippel-lindau disease
well-differentiated liposarcoma
This symptom has already been validated